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Article

Quantifying physical decline in juvenile neuronal ceroid lipofuscinosis (Batten disease).

Neurology - 15 Nov 2011

Kwon J M, Adams H, Rothberg P G, Augustine E F, Marshall F J, Deblieck E A, Vierhile A, Beck C A, Newhouse N J, Cialone J, Levy E, Ramirez-Montealegre D, Dure L S, Rose K R, Mink J W

Abstract excerpt

OBJECTIVE: To use the Unified Batten Disease Rating Scale (UBDRS) to measure the rate of decline in physical and functional capability domains in patients with juvenile neuronal ceroid lipofuscinosis (JNCL) or Batten disease, a neurodegenerative lysosomal storage disorder. We have evaluated the UBDRS in subjects with JNCL since 2002; during that time, the scale has been refined to improve reliability and...

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Identifiers

PMID: 22013180
DOI: 10.1212/WNL.0b013e318237f649
PMCID: PMC3233207

Genes

CLN3

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Source receipt: pubmed, retrieved 7 Sept 2026.

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